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Recurrent Childhood Central Nervous System Embryonal Neoplasm

MedGen UID:
857351
•Concept ID:
C3897757
•
Neoplastic Process

Definition

The reemergence of childhood central nervous system embryonal neoplasm after a period of remission. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRecurrent Childhood Central Nervous System Embryonal Neoplasm

Professional guidelines

PubMed

EANO guideline on rational molecular testing of gliomas, glioneuronal, and neuronal tumors in adults for targeted therapy selection.
Capper D, Reifenberger G, French PJ, Schweizer L, Weller M, Touat M, Niclou SP, Euskirchen P, Haberler C, Hegi ME, Brandner S, Le Rhun E, Rudà R, Sanson M, Tabatabai G, Sahm F, Wen PY, Wesseling P, Preusser M, van den Bent MJ
Neuro Oncol 2023 May 4;25(5):813-826. doi: 10.1093/neuonc/noad008. PMID: 36632791Free PMC Article
Childhood-onset craniopharyngioma: latest insights into pathology, diagnostics, treatment, and follow-up.
Bogusz A, Müller HL
Expert Rev Neurother 2018 Oct;18(10):793-806. Epub 2018 Oct 4 doi: 10.1080/14737175.2018.1528874. PMID: 30257123
Risk-adapted treatment and follow-up management in childhood-onset craniopharyngioma.
Müller HL
Expert Rev Neurother 2016 May;16(5):535-48. Epub 2016 Mar 28 doi: 10.1586/14737175.2016.1166959. PMID: 26982163

Recent clinical studies

Etiology

Pediatric low-grade glioma: State-of-the-art and ongoing challenges.
Fangusaro J, Jones DT, Packer RJ, Gutmann DH, Milde T, Witt O, Mueller S, Fisher MJ, Hansford JR, Tabori U, Hargrave D, Bandopadhayay P
Neuro Oncol 2024 Jan 5;26(1):25-37. doi: 10.1093/neuonc/noad195. PMID: 37944912Free PMC Article
EANO guideline on rational molecular testing of gliomas, glioneuronal, and neuronal tumors in adults for targeted therapy selection.
Capper D, Reifenberger G, French PJ, Schweizer L, Weller M, Touat M, Niclou SP, Euskirchen P, Haberler C, Hegi ME, Brandner S, Le Rhun E, Rudà R, Sanson M, Tabatabai G, Sahm F, Wen PY, Wesseling P, Preusser M, van den Bent MJ
Neuro Oncol 2023 May 4;25(5):813-826. doi: 10.1093/neuonc/noad008. PMID: 36632791Free PMC Article
Anti-GD2 Directed Immunotherapy for High-Risk and Metastatic Neuroblastoma.
Chan GC, Chan CM
Biomolecules 2022 Feb 24;12(3) doi: 10.3390/biom12030358. PMID: 35327550Free PMC Article
Temozolomide with irinotecan versus temozolomide, irinotecan plus bevacizumab for recurrent medulloblastoma of childhood: Report of a COG randomized Phase II screening trial.
Levy AS, Krailo M, Chi S, Villaluna D, Springer L, Williams-Hughes C, Fouladi M, Gajjar A
Pediatr Blood Cancer 2021 Aug;68(8):e29031. Epub 2021 Apr 12 doi: 10.1002/pbc.29031. PMID: 33844469Free PMC Article
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article

Diagnosis

EANO guideline on rational molecular testing of gliomas, glioneuronal, and neuronal tumors in adults for targeted therapy selection.
Capper D, Reifenberger G, French PJ, Schweizer L, Weller M, Touat M, Niclou SP, Euskirchen P, Haberler C, Hegi ME, Brandner S, Le Rhun E, Rudà R, Sanson M, Tabatabai G, Sahm F, Wen PY, Wesseling P, Preusser M, van den Bent MJ
Neuro Oncol 2023 May 4;25(5):813-826. doi: 10.1093/neuonc/noad008. PMID: 36632791Free PMC Article
Amplification of the PLAG-family genes-PLAGL1 and PLAGL2-is a key feature of the novel tumor type CNS embryonal tumor with PLAGL amplification.
Keck MK, Sill M, Wittmann A, Joshi P, Stichel D, Beck P, Okonechnikow K, Sievers P, Wefers AK, Roncaroli F, Avula S, McCabe MG, Hayden JT, Wesseling P, Øra I, Nistér M, Kranendonk MEG, Tops BBJ, Zapotocky M, Zamecnik J, Vasiljevic A, Fenouil T, Meyronet D, von Hoff K, Schüller U, Loiseau H, Figarella-Branger D, Kramm CM, Sturm D, Scheie D, Rauramaa T, Pesola J, Gojo J, Haberler C, Brandner S, Jacques T, Sexton Oates A, Saffery R, Koscielniak E, Baker SJ, Yip S, Snuderl M, Ud Din N, Samuel D, Schramm K, Blattner-Johnson M, Selt F, Ecker J, Milde T, von Deimling A, Korshunov A, Perry A, Pfister SM, Sahm F, Solomon DA, Jones DTW
Acta Neuropathol 2023 Jan;145(1):49-69. Epub 2022 Nov 27 doi: 10.1007/s00401-022-02516-2. PMID: 36437415Free PMC Article
Revived Attention for Adult Craniopharyngioma.
Mende KC, Pantel TF, Flitsch J
Exp Clin Endocrinol Diabetes 2021 Mar;129(3):172-177. Epub 2020 Aug 4 doi: 10.1055/a-1217-7282. PMID: 32750720
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article
Childhood-onset craniopharyngioma: latest insights into pathology, diagnostics, treatment, and follow-up.
Bogusz A, Müller HL
Expert Rev Neurother 2018 Oct;18(10):793-806. Epub 2018 Oct 4 doi: 10.1080/14737175.2018.1528874. PMID: 30257123

Therapy

Intraventricular B7-H3 CAR T Cells for Diffuse Intrinsic Pontine Glioma: Preliminary First-in-Human Bioactivity and Safety.
Vitanza NA, Wilson AL, Huang W, Seidel K, Brown C, Gustafson JA, Yokoyama JK, Johnson AJ, Baxter BA, Koning RW, Reid AN, Meechan M, Biery MC, Myers C, Rawlings-Rhea SD, Albert CM, Browd SR, Hauptman JS, Lee A, Ojemann JG, Berens ME, Dun MD, Foster JB, Crotty EE, Leary SES, Cole BL, Perez FA, Wright JN, Orentas RJ, Chour T, Newell EW, Whiteaker JR, Zhao L, Paulovich AG, Pinto N, Gust J, Gardner RA, Jensen MC, Park JR
Cancer Discov 2023 Jan 9;13(1):114-131. doi: 10.1158/2159-8290.CD-22-0750. PMID: 36259971Free PMC Article
Anti-GD2 Directed Immunotherapy for High-Risk and Metastatic Neuroblastoma.
Chan GC, Chan CM
Biomolecules 2022 Feb 24;12(3) doi: 10.3390/biom12030358. PMID: 35327550Free PMC Article
Locoregional infusion of HER2-specific CAR T cells in children and young adults with recurrent or refractory CNS tumors: an interim analysis.
Vitanza NA, Johnson AJ, Wilson AL, Brown C, Yokoyama JK, Künkele A, Chang CA, Rawlings-Rhea S, Huang W, Seidel K, Albert CM, Pinto N, Gust J, Finn LS, Ojemann JG, Wright J, Orentas RJ, Baldwin M, Gardner RA, Jensen MC, Park JR
Nat Med 2021 Sep;27(9):1544-1552. Epub 2021 Jul 12 doi: 10.1038/s41591-021-01404-8. PMID: 34253928
Temozolomide with irinotecan versus temozolomide, irinotecan plus bevacizumab for recurrent medulloblastoma of childhood: Report of a COG randomized Phase II screening trial.
Levy AS, Krailo M, Chi S, Villaluna D, Springer L, Williams-Hughes C, Fouladi M, Gajjar A
Pediatr Blood Cancer 2021 Aug;68(8):e29031. Epub 2021 Apr 12 doi: 10.1002/pbc.29031. PMID: 33844469Free PMC Article
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article

Prognosis

EANO guideline on rational molecular testing of gliomas, glioneuronal, and neuronal tumors in adults for targeted therapy selection.
Capper D, Reifenberger G, French PJ, Schweizer L, Weller M, Touat M, Niclou SP, Euskirchen P, Haberler C, Hegi ME, Brandner S, Le Rhun E, Rudà R, Sanson M, Tabatabai G, Sahm F, Wen PY, Wesseling P, Preusser M, van den Bent MJ
Neuro Oncol 2023 May 4;25(5):813-826. doi: 10.1093/neuonc/noad008. PMID: 36632791Free PMC Article
Temozolomide with irinotecan versus temozolomide, irinotecan plus bevacizumab for recurrent medulloblastoma of childhood: Report of a COG randomized Phase II screening trial.
Levy AS, Krailo M, Chi S, Villaluna D, Springer L, Williams-Hughes C, Fouladi M, Gajjar A
Pediatr Blood Cancer 2021 Aug;68(8):e29031. Epub 2021 Apr 12 doi: 10.1002/pbc.29031. PMID: 33844469Free PMC Article
Revived Attention for Adult Craniopharyngioma.
Mende KC, Pantel TF, Flitsch J
Exp Clin Endocrinol Diabetes 2021 Mar;129(3):172-177. Epub 2020 Aug 4 doi: 10.1055/a-1217-7282. PMID: 32750720
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article
Risk-adapted treatment and follow-up management in childhood-onset craniopharyngioma.
Müller HL
Expert Rev Neurother 2016 May;16(5):535-48. Epub 2016 Mar 28 doi: 10.1586/14737175.2016.1166959. PMID: 26982163

Clinical prediction guides

EANO guideline on rational molecular testing of gliomas, glioneuronal, and neuronal tumors in adults for targeted therapy selection.
Capper D, Reifenberger G, French PJ, Schweizer L, Weller M, Touat M, Niclou SP, Euskirchen P, Haberler C, Hegi ME, Brandner S, Le Rhun E, Rudà R, Sanson M, Tabatabai G, Sahm F, Wen PY, Wesseling P, Preusser M, van den Bent MJ
Neuro Oncol 2023 May 4;25(5):813-826. doi: 10.1093/neuonc/noad008. PMID: 36632791Free PMC Article
Temozolomide with irinotecan versus temozolomide, irinotecan plus bevacizumab for recurrent medulloblastoma of childhood: Report of a COG randomized Phase II screening trial.
Levy AS, Krailo M, Chi S, Villaluna D, Springer L, Williams-Hughes C, Fouladi M, Gajjar A
Pediatr Blood Cancer 2021 Aug;68(8):e29031. Epub 2021 Apr 12 doi: 10.1002/pbc.29031. PMID: 33844469Free PMC Article
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article
Childhood-onset craniopharyngioma: latest insights into pathology, diagnostics, treatment, and follow-up.
Bogusz A, Müller HL
Expert Rev Neurother 2018 Oct;18(10):793-806. Epub 2018 Oct 4 doi: 10.1080/14737175.2018.1528874. PMID: 30257123
Recurrent pediatric central nervous system low-grade gliomas: the role of surveillance neuroimaging in asymptomatic children.
Udaka YT, Yeh-Nayre LA, Amene CS, VandenBerg SR, Levy ML, Crawford JR
J Neurosurg Pediatr 2013 Feb;11(2):119-26. Epub 2012 Nov 16 doi: 10.3171/2012.10.PEDS12307. PMID: 23157391

Recent systematic reviews

Brain and Brain Stem Necrosis After Reirradiation for Recurrent Childhood Primary Central Nervous System Tumors: A PENTEC Comprehensive Review.
Ajithkumar T, Avanzo M, Yorke E, Tsang DS, Milano MT, Olch AJ, Merchant TE, Dieckmann K, Mahajan A, Fuji H, Paulino AC, Timmermann B, Marks LB, Bentzen SM, Jackson A, Constine LS
Int J Radiat Oncol Biol Phys 2024 Jun 1;119(2):655-668. Epub 2024 Feb 1 doi: 10.1016/j.ijrobp.2023.12.043. PMID: 38300187

Supplemental Content

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